Table 1.
Family | Ethnicity | Gene | Mutation (nt) LRTOMT2a | Mutation (aa) LRTOMT2 | Mutation (nt) LRTOMT1a | Mutation (aa) LRTOMT1 | Allele frequencyb |
---|---|---|---|---|---|---|---|
TR57 | Turkish | LRTOMT | c.358+4G>A | p.A29SfsX54 | c.761+4G>A | p.G163VfsX4 | 0/176 |
FT1A-G | Tunisian | LRTOMT | c.242G>A | p.R81Q | c.645G>A | p.A215A | 0/190 |
FT2 | Tunisian | LRTOMT | c.313T>C | p.W105R | c.716T>C | 3’ UTR | 0/180 |
PKDF702 | Pakistani | LRTOMT | c.328G>A | p.E110K | c.731G>A | 3’ UTR | 0/364 |
PKDF537 | Pakistani | * |
Nucleotide changes are numbered according to the first coding ATG (Accession numbers EU627069, EU627070).
Ethnically matched hearing subjects.
In family PKDF537, no pathogenic mutations were found either in the coding region of FGF3, LRTOMT or in 25 evolutionary conserved regions in the introns of LRTOMT, suggesting that the mutation might be in a nonconserved region of an intron, in a distant regulatory element, or in another gene in the DFNB63 interval. Alternatively, the hearing loss segregating in this large family may have been spuriously linked to chromosome 11q13.3 despite a LOD score of 6.98.